Nishie Makoto, Mori Fumiaki, Fujiwara Hideo, Hasegawa Masato, Yoshimoto Makoto, Iwatsubo Takeshi, Takahashi Hitoshi, Wakabayashi Koichi
Department of Neuropathology, Institute of Brain Science, Hirosaki University School of Medicine, 5 Zaifu-cho, 036-8562, Hirosaki, Japan.
Acta Neuropathol. 2004 Apr;107(4):292-8. doi: 10.1007/s00401-003-0811-1. Epub 2004 Jan 14.
We immunohistochemically examined the brain and peripheral sympathetic ganglia from eight patients with multiple system atrophy (MSA), using an antibody specific for phosphorylated alpha-synuclein (anti-PSer129). Phosphorylated alpha-synuclein was deposited in five cellular locations: oligodendroglial cytoplasm and nucleus, and neuronal cytoplasm, processes and nucleus. Many neuronal cytoplasmic inclusions (NCIs) were found in the pontine and inferior olivary nuclei and, to a lesser extent, in the substantia nigra, locus ceruleus, and neocortical and hippocampal neurons. NCIs were also found in the sympathetic ganglia in two out of the eight cases. Moreover, anti-PSer129 immunohistochemistry revealed extensive neuropil pathology; swollen neurites were abundant in the pontine nucleus, delicate neurites were observed in the deeper layers of the cerebral cortex and thalamus, and neuropil threads and dot-like structures were distributed in the basal ganglia and brainstem. Diffuse neuronal cytoplasmic staining (pre-NCI) was frequently found in the pontine and inferior olivary nuclei. Thus, the widespread accumulation of phosphorylated alpha-synuclein in both glial and neuronal cells is a pathological feature in patients suffering from MSA.
我们使用针对磷酸化α-突触核蛋白的特异性抗体（抗PSer129），对8例多系统萎缩（MSA）患者的脑和外周交感神经节进行了免疫组织化学检查。磷酸化α-突触核蛋白沉积在五个细胞位置：少突胶质细胞的细胞质和细胞核、神经元的细胞质、突起和细胞核。在脑桥核和下橄榄核中发现了许多神经元胞质内包涵体（NCI），在黑质、蓝斑以及新皮质和海马神经元中也有较少发现。在8例中的2例交感神经节中也发现了NCI。此外，抗PSer129免疫组织化学显示广泛的神经纤维病理改变；脑桥核中可见大量肿胀的神经突，大脑皮质深层和丘脑可见纤细的神经突，基底节和脑干中有神经纤维丝和点状结构分布。在脑桥核和下橄榄核中经常发现弥漫性神经元胞质染色（前NCI）。因此，磷酸化α-突触核蛋白在胶质细胞和神经元细胞中的广泛积累是MSA患者的一个病理特征。
